Canakinumab treatment real world evidence in 3 monogenic periodic fever syndromes in 2009-2022: an interim analysis using the French JIR cohort database.

Background: Our study aimed to provide real-world evidence on the treatment patterns, effectiveness and safety of canakinumab in France in Familial Mediterranean Fever (FMF), Mevalonate Kinase Deficiency (MKD), and Tumor necrosis factor Receptor Associated Periodic Syndrome (TRAPS).

Methods: This study used the JIR cohort, a multicentre international registry created in 2013 to collect data on patients with juvenile inflammatory rheumatic diseases. French patients diagnosed with FMF, MKD or TRAPS and treated with canakinumab were included in this study.

Long-term results of Waldenström macroglobulinaemia treatment by bendamustine and rituximab: A study on behalf of the French Innovative Leukemia Organization (FILO).

The bendamustine-rituximab (BR) schedule is an efficient first-line therapy in Waldenström macroglobulinaemia (WM). A previous analysis of 69 patients who received this treatment confirmed a high response rate and good progression-free (PFS) and overall survival (OS). With a median follow-up of 76.

Respiratory Infection Triggering Severe Acute Exacerbations of Chronic Obstructive Pulmonary Disease.

Background: Data are scarce on respiratory infections during severe acute exacerbation of chronic obstructive pulmonary disease (COPD). This study aimed to investigate respiratory infection patterns in the intensive care unit (ICU) and identify variables associated with infection type and patient outcome.

Methods: A retrospective, single-centre cohort study.

Proposition of a transdiagnostic processual approach of emotion dysregulation based on core triggers and interpersonal styles.

Emotion dysregulation (ED) has primarily been described in patients suffering from borderline personality disorder (BPD) and is an integral part of this diagnosis, but it is also a transdiagnostic construct that can be found in several other psychiatric disorders. The strong relationships between ED and BPD may lead clinicians to underestimate ED associated to other clinical contexts. This can lead to difficulties in diagnostic and treatment orientation, especially in the context of comorbidities.

Evolution of REperfusion Strategies and impact on mortality in Old and Very OLD STEMI patients. The RESOVOLD-e-MUST study.

Background: The use of myocardial reperfusion-mainly via angioplasty-has increased in our region to over 95%. We wondered whether old and very old patients have benefited from this development.

Methods: Setting: Greater Paris Area (Ile-de-France).

A20 Haploinsufficiency: A Systematic Review of 177 Cases.

A20 haploinsufficiency is an autoinflammatory disease caused by defective inactivation of the NF-κB pathway. We conducted a systematic literature review of articles reporting patients with TNFAIP3 sequence variants from 2016 to August 2023 following PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analyses) guidelines. Data from 177 patients from 65 articles were retrieved (108 women).

Clinical and cognitive characteristics of subjects with schizophrenia and childhood attention-deficit/hyperactivity disorder: Results from the multicentric FACE-SZ cross-sectional dataset.

Background: Childhood Attention-deficit/hyperactivity disorder (C-ADHD) is a neurodevelopmental disorder, associated with an increased risk of subsequent schizophrenia. The objective of the present study was to determine the prevalence of C-ADHD in schizophrenia and the clinical and cognitive characteristics associated with C-ADHD history in schizophrenia.

Methods: 569 subjects with schizophrenia (74 % men, mean age 30.

Predictors of medication adherence in a large 1-year prospective cohort of individuals with schizophrenia: insights from the multicentric FACE-SZ dataset.

Schizophrenia is characterized by the most salient medication adherence problems among severe mental disorders, but limited prospective data are available to predict and improve adherence in this population. This investigation aims to identify predictors of medication adherence over a 1-year period in a large national cohort using clustering analysis. Outpatients were recruited from ten Schizophrenia Expert Centers and were evaluated with a day-long standardized battery including clinician and patient-rated medication adherence measures.

Long-term heart function in cardiac-arrest survivors.

Purpose: To assess outcomes and predictors of long-term myocardial dysfunction after cardiac arrest (CA) of cardiac origin.

Methods: We retrospectively included consecutive, single-center, prospective-registry patients who survived to hospital discharge for adult out-of-hospital and in-hospital CA of cardiac origin in 2005-2019. The primary objective was to collect the 1-year New York Heart Association Functional Class (NYHA-FC) and major adverse cardiovascular events (MACE).

Treat-to-target strategies for the management of familial Mediterranean Fever in children.

Background: The objective of this initiative was to develop a treat-to-target (T2T) approach for the management of patients with Familial Mediterranean Fever (FMF), including the definition of a complex treatment target, and establish strategies that improve patient care and long-term outcome.

Methods: An initial set of statements as well as a flow chart visualising the proposed concept was developed. To adapt the preliminary statements to the current state of knowledge, a systematic literature search was performed and the modified statements were subject to a Delphi approach.

Evaluation of mechanical thrombectomy in acute ischemic stroke related to a distal arterial occlusion: A randomized controlled trial.

Rationale: Mechanical thrombectomy (MT) associated with the best medical treatment (BMT) has recently shown efficacy for the management of acute ischemic stroke (AIS) secondary to a large vessel occlusion. However, evidence is lacking regarding the benefit of MT for more distal occlusions.

Aim: To evaluate the efficacy in terms of good clinical outcome at 3 months of MT associated with the BMT over the BMT alone in AIS related to a distal occlusion.

Clinical, imaging, and management features of symptomatic carotid web: Insight from CAROWEB registry.

Background: Although carotid web (CaW) is increasingly diagnosed as a cause of cryptogenic stroke, data are still limited to monocentric small sample cohort. To broaden knowledge on symptomatic CaW, CAROWEB registry has been recently implemented.

Aims: In a large cohort of symptomatic CaW patients, we described epidemiologic characteristics, admission clinical and imaging features, and the current management including the secondary preventive strategy choice made in comprehensive French Stroke Units.

Anterior Skull Base Abnormalities in Congenital Anosmia.

Introduction: The structures of the skull and the brain are related to each other. Prior work in individuals with isolated congenital anosmia (ICA) showed that these individuals were characterized by olfactory bulb (OB) defects. The aim of this study was to compare the morphological pattern of the anterior skull base surrounding the OB between individuals with ICA and normosmic controls.

Case reports: Using Good Psychiatric Management (GPM) conceptualizations in the dimensional assessment and treatment of personality disorders.

Good Psychiatric Management (GPM) is a generalist clinical management approach for borderline personality disorder that incorporates common ingredients of good standard care for any psychiatric diagnosis with what works from prevailing specialist psychotherapies. Similar to all validated therapies for BPD, it relies on a specified formulation of the disorder' symptoms as arising from , to dynamically describe typical patterns of daily self- and interpersonal issues that drive the instability that defines the general personality dysfunction characteristic of the disorder. Recent adaptations of GPM have been proposed for narcissistic personality disorder and obsessive-compulsive personality disorder, with development of similar dynamic models for both ( and ).

Breaking down the fences among registries on autoinflammatory diseases: the E-Merge project.

Background: Among the various numbers of different autoinflammatory diseases (AIDs), the absolute majority of them remains rare, with a single representative in large populations. This project, endorsed by PRES, supported by the EMERGE fellowship program, and performed in line with the Metadata registry for the ERN RITA (MeRITA), has the objective of performing a data synchronization attempt of the most relevant research questions regarding clinical features, diagnostic strategies, and optimal management of autoinflammatory diseases.

Results: An analysis of three large European registries: Eurofever, JIR-cohort and AID-Net, with a total coverage of 7825 patients from 278 participating centers from different countries, was performed in the context of epidemiological and clinical data merging.

Multisystem inflammatory syndrome in children during the COVID-19 waves: data from the Juvenile Inflammatory Rheumatism cohort.

Introduction: Multisystem inflammatory syndrome in children (MIS-C) is a new condition that first appeared in children and adolescents during the COVID-19 pandemic. We aimed to describe the diagnostic course, clinical and biological manifestations, and treatment of MIS-C during the first three COVID-19 waves.

Methods: We extracted patient data from the Juvenile Inflammatory Rheumatism (JIR) cohort.

Unchanged Characteristics and Survival among Critically Ill COVID-19 Patients during First, Second, and Third Waves: A Prospective Observational Cohort.

Background: This study was carried out to compare characteristics and outcomes in patients with acute respiratory failure related to COVID-19 during first, second, and third waves.

Methods: We included consecutive adults admitted to the intensive care unit between March 2020 and July 2021. We compared three groups defined by the epidemic intake phase: waves 1 (W1), 2 (W2), and 3 (W3).

Observational Cohort Study Dedicated to Autism Spectrum Disorder: Milestone Steps, Results Updates, Perspectives.

Autism Spectrum Disorder (ASD) is a neurodevelopmental condition characterized by persistent difficulties in two domains: social communication and interaction, alongside with restricted, repetitive pattern of behaviors. It affects children and persists into adolescence and adulthood. Its causes and underlying psychopathological mechanisms are unknown and remain to be discovered.